Stock 129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH
FESA Number
1974
Strain Name
129.C-Tg(Prnp/PRNP)1Drb 129-Prnp<tm1Cwe>/DrbH
Mutation Strain Type
Attribution
Gene/Allele Information
| Allele Name | Allele MGI ID | Gene Name | Gene MGI ID | Chromosome |
|---|---|---|---|---|
| Prnp<tm1Cwe> | MGI:1888773 | Prnp | MGI:97769 | 2 |
Phenotypic Description
The octameric repeat region of the mouse prion protein was replaced with the hexameric repeat region from the domestic chicken. The mice express mouse PrP at wild-type levels with correct membrane location and orientation but have an altered metal binding region. The mice have been generated to get a better understanding of prion diseases such as CJD in humans.
Orphanet Disorders
| OrphaNet ID | Name | Url |
|---|---|---|
| 356 | Gerstmann-Straussler-Scheinker syndrome | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=356 |
| 466 | Fatal familial insomnia | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=466 |
| 157 941 | Huntington disease-like 1 | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=157941 |
| 280 397 | Familial Alzheimer-like prion disease | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=280397 |
| 282 166 | Inherited Creutzfeldt-Jakob disease | http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=282166 |
Publications
Displaying 31 - 40 of 226 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 24329154 | Alvarez-Laviada A | The inhibition of functional expression of calcium channels by prion protein demonstrates competition with ?2? for GPI-anchoring pathways. | Biochem J (2 014) 458:365-74 |
| 22428057 | Biasini E | The toxicity of a mutant prion protein is cell-autonomous, and can be suppressed by wild-type prion protein on adjacent cells. | PLoS One (2 012) 7:e33472 |
| 20472884 | Christensen HM | A highly toxic cellular prion protein induces a novel, nonapoptotic form of neuronal death. | Am J Pathol (2 010) 176:2695-706 |
| 16621165 | Diez M | Changes in neuropeptide expression in mice infected with prions. | Neurobiol Aging (2 007) 28:748-65 |
| 16738485 | Gains MJ | Prion protein protects against ethanol-induced Bax-mediated cell death in vivo. | Neuroreport (2 006) 17:903-6 |
| 17420473 | Huang S | Inducible overexpression of wild-type prion protein in the muscles leads to a primary myopathy in transgenic mice. | Proc Natl Acad Sci U S A (2 007) 104:6800-5 |
| 12878182 | Kubosaki A | Expression of normal cellular prion protein (PrP(c)) on T lymphocytes and the effect of copper ion: Analysis by wild-type and prion protein gene-deficient mice. | Biochem Biophys Res Commun (2 003) 307:810-3 |
| 15694261 | Lobão-Soares B | Normal brain mitochondrial respiration in adult mice lacking cellular prion protein. | Neurosci Lett (2 005) 375:203-6 |
| 18514536 | Medrano AZ | GFP-tagged mutant prion protein forms intra-axonal aggregates in transgenic mice. | Neurobiol Dis (2 008) 31:20-32 |
| 22860039 | Passet B | Prion protein and Shadoo are involved in overlapping embryonic pathways and trophoblastic development. | PLoS One (2 012) 7:e41959 |
Pages
Orphanet Categories
Rare neurologic disease
EMMA ID
EM:02421
Genetic Status
GMO
Background Strain Name
129
Background Strain MGI ID
MGI:2160041
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